Rheumatologic diseases have varied clinical presentations, and posterior reversible encephalopathy syndrome (PRES) can be one of their presentations. The exact etiology of PRES is unknown, but endothelial dysfunction and immunosuppressive medications seem to be the likely cause in rheumatologic diseases. Clinical features include headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. The diagnosis of PRES can be difficult because several neuropsychiatric illnesses are generally prevalent in rheumatologic diseases; however, a high index of suspicion among physicians along with neuroimaging can help in the accurate diagnosis. Treatment guidelines are lacking, but in a few case series, lowering the blood pressure, controlling the seizures, and removing the immunosuppressive drugs have shown good results. There is need for randomized controlled trials addressing the treatment of PRES in rheumatologic diseases. Medline search was done from year 1950 to March 2011 using "posterior reversible encephalopathy" as keyword, and articles relevant to rheumatology were reviewed. We found 48 case reports showing PRES in patients with rheumatologic disease. Most of the patients were female. Age range was from 6 to 59 years. Out of the 48 case reports, 38 patients had systemic lupus erythematosus and most of them had renal disease. Five patients with autoimmune diseases presented with PRES after being started on immunomodulatory drugs. The most frequent symptoms were headache, seizures, and visual changes.
Patient: Female, 67Final Diagnosis: GPA and GCASymptoms: Blurry vision • headache • nosebleed • sinus congestionMedication: —Clinical Procedure: —Specialty: RheumatologyObjective:Rare co-existance of disease or pathologyBackground:Systemic vasculitis can present with a multitude of symptoms involving multiple organ systems. Clinicians should avoid anchoring bias and be cognizant that different types of vasculitides can be present in the same patient and that the diagnosis of one should not preclude the subsequent diagnosis of another.Case Report:A 67-year-old woman was referred for evaluation of episodes of epistaxis and recurrent severe sinusitis. Her physical examination showed nasal congestion and purpuric rash on the lower extremities. CT of the sinuses showed severe mucosal thickening. ANCA serologies were positive with a c-ANCA titer of 1: 5120 and anti-proteinase-3 (anti-PR3) antibodies of 1061 units. Serum creatinine was elevated at 1.32 mg/dL (GFR of 40.62 ml/min). Urine analysis showed proteinuria and hematuria. The patient declined treatment initially, but while awaiting kidney biopsy she developed episodes of headache and blurry vision. She underwent right temporal artery biopsy 4 days later, which confirmed the diagnosis of GCA. The biopsy showed characteristic histopathology findings and she was started on 60 mg of prednisone daily. The kidney biopsy showed pauci-immune crescentic glomerulonephritis (PICGN) consistent with ANCA-associated vasculitis. We identified all the cases of co-presentation of GCA and GPA in the literature and summarized their clinical features in this report.Conclusions:Astute clinicians should be cognizant of overlapping and atypical presentations of vasculitides to avoid delayed diagnosis and errors in management.
Physician-patient email communication is gaining popularity. However, a formal assessment of physicians' email communication skills has not been described. We hypothesized that the email communication skills of rheumatology fellows can be measured in an objective structured clinical examination (OSCE) setting using a novel email content analysis instrument which has 18 items. During an OSCE, we asked 50 rheumatology fellows to respond to a simulated patient email. The content of the responses was assessed using our instrument. The majority of rheumatology fellows wrote appropriate responses scoring a mean (±SD) of 10.6 (±2.6) points (maximum score 18), with high inter-rater reliability (0.86). Most fellows were concise (74%) and courteous (68%) but not formal (22%). Ninety-two percent of fellows acknowledged that the patient's condition required urgent medical attention, but only 30% took active measures to contact the patient. No one encrypted their messages. The objective assessment of email communication skills is possible using simulated emails in an OSCE setting. The variable email communication scores and incidental patient safety gaps identified, suggest a need for further training and defined proficiency standards for physicians' email communication skills.
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